Multiple cutaneous granular cell tumors of the scrotum

A 55-year-old black man with two firm intradermal nodules of the scrotum is presented. On histopathologic examination, the nodules were determined to be cutaneous granular cell tumors. This was confirmed by strong immunohistochemical reactivity to S-100 protein. There are no previous reports of multiple granular cell tumors of the male genitalia. Only one report of a solitary granular cell tumor of the scrotum appears in the literature.     

Multiple cutaneous reticulohistiocytosis

BACKGROUND: Multicentric reticulohistiocytosis is a non Langerhans cell histiocytosis. This rare disease is characterized by cutaneous papules and nodules and a destructive polyarthritis; multisystem involvement may occur. Multiple and diffuse cutaneous reticulohistiocytosis have been more rarely reported. We present a case which was distinctive by the existence of multiple cutaneous plaques. CASE REPORT: A 65-year-old woman presented cutaneous papules and nodules associated with a destructive arthritis affecting the hands. Histological examination of a cutaneous biopsy associated with immunophenotyping and electronic microscopy permitted us to make the diagnosis of multicentric reticulohistiocytosis. The search for visceral involvement or underlying neoplasia was negative. Rapidly, cutaneous aggravation occurred with multiple and diffuse infiltrated plaques on the back, the face, the ears, the thighs and the forearms. The same histological aspect was found for these lesions. Treatment with corticosteroids and cyclophosphamide was successful. DISCUSSION: This case report is the first one with diffuse cutaneous lesions of multicentric reticulohistiocytosis with aspect of infiltrated plaques. Diffuse cutaneous lesions in multicentric reticulohistiocytosis have been rarely reported with diffuse papules or nodules pattern. A visceral involvement seems to be more frequent for diffuse cutaneous involvement. In all cases, the association of multicentric reticulohistiocytosis with neoplasia in up to 25 p. 100 is of interest. Treatment of multicentric reticulohistiocytosis consists in corticosteroids at the initial phase associated with alkylants agents or methotrexate.     

Multiple cutaneous myxomas

An 11-year-old Hispanic female was referred for evaluation of multiple asymptomatic papules which had slowly developed since age two. The patient had no significant medical history and no family members, including an older sibling, were similarly affected. Physical examination revealed multiple 2–6 mm flesh-colored to erythematous papules over the posterior scalp, trunk, and extremities, predominantly in a midline distribution. No lentigines, blue nevi, or evidence of endocrine ab-normalities were noted. Echocardiogram was normal with no evidence of myxoma. Six cutaneous biopsies showed myxomas, as evidenced by well-circumscribed dermal tumors composed of stellate, dendritic cells embedded in a mucinous stroma. Special stains and electron microscopy confirmed the H&E findings. Immunoperoxidase staining for Factor XIIIa was positive in the dendritic tumor cells, suggesting a relationship with dermal dendrocytes. This patient represents a case of multiple cutaneous myxomas without other components of Carney's complex of myxomas, spotty pigmentation, endocrine overactivity, and a variety of uncommon tumors. A long-term follow-up is indicated since some of the above cited elements of the syndrome could appear over time.     

Multiple cutaneous leiomyomata

This is a report of two cases of multiple cutaneous leiomyomata both having a past history of uterine leiomyomata requiring hysterectomy. Two sisters of one of the patients are known to suffer from cutaneous leiomyomata and one of them has also had an hysterectomy for fibroids. The association of cutaneous and uterine leiomyomata has been previously reported and the question of a real or fortuitous connection between these two types of smooth-muscle tumour has been raised.     

Multiple cutaneous granular cell tumors: a case report and review of the literature

Granular cell tumors are benign neoplasms derived from Schwann cells. Their clinical presentation ranges from large verrucose nodules, as in our patient, to small, nonspecific, subcutaneous papules. Approximately half of granular cell tumors develop in the head-and-neck region, most commonly on the tongue. Multiple granular cell tumors are not a sign of malignancy and may be more common than generally reported.     

Multiple cutaneous melanoma metastases

An 84-year-old white woman presented with diagnosed vascular failure in the left leg. The patient had had diabetes mellitus for over 50 years. Five years earlier, her right leg was amputated because of an obliterating arteriopathy. At the time of hospitalization, she showed dry gangrene in all the toes on her left foot. A laser Doppler test performed on the left leg showed severe peripheral arteriopathy affecting the anterior and posterior tibial arteries, with a Windsor index of 20% in her ankle. On physical examination, the patient showed several nodular lesions on the anterior and anterior-medial surface of her left leg. These lesions were infiltrated, of a hard elastic consistency, and occasionally confluent, with a diameter ranging from 0.5 to 4 cm (Fig. 1) and the color varying from ochre-yellow to brownish-red to slate-brown. The patient reported that these lesions had erupted approximately 2 years earlier and had been accompanied by itching and mild burning. She had shown them to her family doctor who had diagnosed seborrhoeic keratosis. A series of biopsies performed on the nodules revealed the presence of atypical melanocytes arranged in alveolar formations that reached the epidermis. The epidermis appeared thin, compressed, and atrophic; no signs of junctional activity could be seen (Fig. 2). The tumor cells were round, with a clear cytoplasm and atypical, hyperchromatic, oval nuclei containing nucleoli (Fig. 3). Some atypical melanocytes contained small amounts of melanic pigment that was concentrated in the cytoplasm of macrophages which were mixed with the elements of the tumor. There had been no melanoma in the clinical history of the patient. The patient had a chest roentgenogram with tomography and a computer tomography (CT) scan of the liver, spleen, mediastinum, and brain, all with negative results. The case is worth reporting because of the peculiar cutaneous lesions that could suggest several diagnostic hypotheses. The clinical picture could have been interpreted as being Kaposi's sarcoma, seborrhoeic keratosis, angiosarcoma, or multiple Spitz nevi. A Spitz nevus is a benign variant of a compound nevus that is characterized histopathologically by atypical, large melanocytes with abundant eosinophilic cytoplasm and large vesicular nuclei with prominent nucleoli. Histologic examination removed any doubt, suggesting a metastatic origin for the proliferating nodules. Marked expression of S-100 protein and of HMP45 was found by immunohistochemistry; HMP45 was also intensely positive in the deep layers of the dermis. It was not possible to define the site of the primary lesion from the patient's history or from physical examination;^1,2 however, one cannot exclude that the primary lesion was at the same site as where the metastases had diffused, either by growing spontaneously from healthy tissue or through a transformation of anevus.^3,4     

Multiple familial cutaneous leiomyoma

INTRODUCTION: Cutaneous leiomyoma is a benign tumor, the discovery of which may suggest a hereditary form. We report a family in which 5 generations developed cutaneous and uterine leiomyomas. The originality of this report lies in the large number of generations developing the disease and the association with chronic myeloid leukemia. OBSERVATIONS: We have studied 16 members of a family with cutaneous and uterine leiomyomas spanning five generations. Eight members of the family (6 women and 2 men) presented with cutaneous leiomyomas. All 6 women also had uterine myomas with complications (menometrorrhagia, miscarriage, premature delivery and hysterectomy). Pathological association was also confirmed: polycythemia (1 case), papillary renal carcinoma (1 case) and chronic myeloid leukemia (1 case). DISCUSSION: Piloleiomyoma can develop sporadically or can be transmitted genetically. To our knowledge, we report the fifth case of a family of more than 2 generations presenting with piloleiomyoma. By studying the family tree, we were able to confirm the dominant autosomal nature of the mode of transmission found by other authors. The association of piloleiomyoma and uterine myoma is classified as Reed's syndrome. In such cases, the uterine myoma requires particularly careful monitoring since it is associated with significant risk of gynecological complications (menometrorrhagia, miscarriage, premature delivery and postpartum hemorrhage). Moreover, in our observations we describe diseases associated with piloleiomyoma: polycythemia (1 case), papillary renal carcinoma (1 case), but also the association of piloleiomyoma with chronic myeloid leukemia (1 case). A previous report described the same genetic deletion in uterine myoma as in chronic myeloid leukemia, which gives further weight to this association.     

Multiple cutaneous granular cell tumors: case report of a 19-year-old African American female

BACKGROUND AND OBJECTIVE: This is a somewhat rare case of a 19-year-old African American female with multiple cutaneous granular cell tumors. Granular cell tumors are of neural origin, except in rare cases, and are considered benign, with a low incidence of malignancy. The clinical presentation varies greatly, but these tumors are most commonly painful and slow growing, with two-thirds occurring on the head and neck. Patients are most commonly in their second to fourth decades of life, two-thirds are black, and two-thirds are women. Granular cell tumors are diagnosed by the characteristic pathologic findings of polygonal cells with eosinophilic granular cytoplasm. CONCLUSION:These tumors are most commonly singular but can be multiple in 10 to 15% of patients. Older patient age, rapid growth or enlargement, and a history of local recurrence should raise concern for malignant behavior. The distribution and family history in this case are suggestive of possible mosaicism.     

Multiple cutaneous granular cell tumors, joint hypermobility and mild facial dysmorphism in a child

BACKGROUND: The association of multiple cutaneous granular tumors with systemic defects is extremely rare. To date, 14 cases have been described. CASE REPORT: A 14-year-old boy presented multiple nodular lesions on his arms and legs; facial dysmorphism and joint hypermobility were also present. Histopathologic examination of two nodules showed granular cell tumors. We review the literature regarding multiple granular cell cutaneous tumors in children with associated somatic defects and genetic syndromes. CONCLUSIONS: The combination of multiple cutaneous granular tumors with alterations in other organs is not only fortuitous, justifying the proposal of a distinct syndrome, termed "Bakos's syndrome" after the author who first described this association. The relation with neurofibromatosis type 1 is discussed, and an association is suggested with the range of variants of this syndrome.     

Multiple isolated cutaneous plexiform schwannomas

Plexiform schwannoma is a rare neurogenic tumor, arising from skin and subcutaneous tissue. The presence of multiple schwannomas suggests a possible association with neurofibromatosis type 2 (NF2). A 50-year old male patient presented with multiple papulo-nodular cutaneous lesions on both arms and forearms. Histopathological examination revealed a dermal multinodular pattern of well-circumscribed masses of closely packed cells, with peripheral myxoid tissue, well-encapsulated in a thin collagenous capsule. S-100 immunohistochemical staining was diffusely and strongly positive. Neuron-specific enolase was positive, confirming a neural tissue tumor. An audiogram and Magnetic Resonance Imaging (MRI) of cerebro-pontine angle showed no detected abnormality, excluding acoustic neuroma. Thus, we present a case of multiple bilateral isolated cutaneous plexiform schwannomas, not associated with NF2. Multiple plexiform schwannomas is a very rare entity, distinct from neurofibromatosis (NF), and being confined to the dermis is even more rarely reported.     

Multiple primary cutaneous plasmacytomas.

BACKGROUND--Cutaneous plasmacytoma is an uncommon tumor and is mostly seen in the context of end-stage multiple myeloma. Only 20 cases of primary cutaneous plasmacytoma have been documented. A significant proportion of these patients went on to develop systemic disease with a poor prognosis. In a number of patients, however, the abnormal clone of plasma cells may arise in the skin and never progress to multiple myeloma involving the bone marrow. OBSERVATIONS--We describe a patient who developed multiple primary cutaneous plasmacytomas after a possible insect bite reaction. The monoclonality of the tumor cells is demonstrated using immunohistochemical techniques. He has been treated vigorously with chemotherapy and local radiotherapy and remains well 3 years after diagnosis. Bone marrow has been harvested for use as an autologous bone marrow transplant in the event of systemic relapse. CONCLUSIONS--Unlike previous reports of this rare entity, this case documents the monoclonality of tissue plasma cells with immunohistochemical techniques. As cutaneous plasmacytomas have been reported with an early significant mortality, unlike extramedullary plasmacytomas elsewhere, we have advocated combination chemotherapy and cryopreservation of uninvolved bone marrow for future autologous bone marrow transplantation should systemic myelomatosis develop in the patient.